Because digital subtraction angiography (DSA) is not an ideal angiographic examination for moyamoya disease in the pediatric population, magnetic resonance angiography (MRA) provides a noninvasive contrast-free angiographic examination; whereas magnetic resonance imaging (MRI) provides superior spatial resolution and soft-tissue contrast for lesion assessment. Ninety patients with moyamoya disease were examined by MRI and DSA to assess the distribution of lesions and their diagnostic agreement between modalities. MRI examination revealed 439 lesions. Punctate lesions were the most abundant, followed by patchy lesions. These lesions generally covered a smaller area than the abnormal-vascular corresponding brain parenchyma. Steno-occlusive changes at bilateral anterior, medial, and posterior cerebral arteries were identified by MRA and DSA. MRI showed moderate agreement in identifying lesions after steno-occlusive changes in anterior and medial cerebral arteries, and good agreement in posterior cerebral arteries; 6% to 11% of cases were misdiagnosed by MRA.